This is a fantastic article for anyone with PCOS (or with a daughter or loved one with PCOS). It is VERY in-depth!
Polycystic Ovary Syndrome (PCOS) in the Adolescent Patient: Recommendations for Practice
Barbara S. Snyder
Polycystic ovary syndrome (PCOS) is an endocrine disorder affecting women across the lifespan. Originally thought only to impact women of child-bearing age because of the presence of infertility associated with the disease, health care professionals are now realizing that adolescent females are presenting with PCOS and the associated health concerns of menstrual irregularities, obesity, type 2 diabetes, and evidence of hyperandrogenism (hirsutism and acne) with increasing prevalence. In fact, there seems to be an over-representation of obesity-related type 2 diabetes in adolescents, especially in those females who present with signs of hyperandrogenism and menstrual irregularities (Lewy, Danadian, Witchel, & Arslanian, 2001).
PCOS often manifests around the time of menarche as irregular and often lengthened menstrual cycles (Richardson, 2003). Unfortunately, PCOS often goes unrecognized and undiagnosed at this time because most adolescents do not have regular menstrual cycles (Meisler, 2002). These young women also go undiagnosed because the prescribed treatment for irregular menstrual cycles is the use of oral contraceptive pills (OCPs). OCPs will regulate menstrual cycles and often times control acne and hirsutism. These girls often will not receive a diagnosis until much later, perhaps at the time when they seek treatment for infertility.
The main concerns in caring for the adolescent with PCOS are twofold. The first involves cyclic control of irregular menstruation cycles. By having predictable menstrual cycles, young females would avoid the embarrassment that is often associated with irregularity. Also, because of the irregular nature of the cycles seen in women with PCOS, leading to a span of time between periods of anywhere between 45 days and 365 days, the menstrual flow is heavier and the period is associated with significant cramping (Richardson, 2003). The second issue involves the avoidance of the long-term sequelae that are associated with obesity, insulin resistance, glucose intolerance, and type 2 diabetes. These conditions can result in subsequent lipid abnormalities and hypertension that are significant risk factors in the development of cardiovascular disease.
Because PCOS is a lifelong disorder with significant long-term health risks, nurses working with adolescent patients need to keep this endocrine/metabolic syndrome in mind, especially when a female presents with obesity, acne, hirsutism, and irregular menses. This article will address the following issues related to PCOS in the adolescent patient: (a) pathogenesis of the disorder; (b) recommendations for the diagnostic work-up in terms of history, physical exam, and laboratory testing; (c) recommendations for management strategies that can help alleviate the troubling signs and symptoms with which teens often present; and (d) resources that are available to teens to help them learn more about the disease and get needed psychosocial support. Early intervention through lifestyle modification and the use of various medications is essential to prevent the medical co-morbidities associated with PCOS.
PCOS is a heterogeneous endocrine disorder diagnosed in 5%-10% of women in the U.S. (Markle, 2001; Tweedy, 2000). It is most likely a genetic disorder but can appear in girls with no prior identifiable family history. The most troubling outward signs of the disease involve those that occur as the result of hyperandrogenism. The hyperandrogenism occurs primarily because of an overproduction of testosterone from ovarian thecal cells and the adrenal gland. Hyperandrogenism manifests in females as hirsutism, acne, frontal and temporal balding, deepening voice, increased muscle mass, decreased breast size, and in severe cases, virilization involving clitoromegaly (Marshall, 2001).
The other prominent endocrine component involves the effects of insulin. Women with PCOS tend to be insulin resistant with accompanying hyperinsulinemia. Insulin resistance means that the body tissues do not respond to insulin. To overcome insulin resistance, the body secretes more insulin, thus causing a hyperinsulinemic state (Azziz, 2004). The endocrine problems found in adolescent girls with PCOS include reduced peripheral tissue insulin sensitivity, hepatic insulin resistance, and hyperinsulinemia; these are all predictive of type 2 diabetes (Lewy et al., 2001).
In addition to impacting glucose metabolism, insulin also affects circulating androgen levels. Increased circulating levels of testosterone are noted in women with insulin resistance and hyperinsulinemia. This occurs because high levels of insulin decrease circulating levels of sex hormone binding globulin (SHBG). This in turn leads to increasing levels of free testosterone and a worsening of the signs of hyperandrogenism (Azziz, 2004). These increased levels of androgens are not only responsible for hirsutism, acne, and male pattern baldness but also the development of central/android obesity as well. The presence of this central/android obesity only serves to aggravate insulin resistance, thereby worsening PCOS symptoms.
Obesity, insulin resistance, hyperinsulinemia, glucose intolerance, and type 2 diabetes have a great impact on overall health, particularly the development of heart disease. Women with PCOS who manifest these endocrine disturbances have been shown to have increased blood pressure, increased low-density lipoprotein (LDL) cholesterol, and decreased high-density lipoprotein (HDL) cholesterol (Sheehan, 2004). All of these are known to be significant risk factors for the development of cardiovascular disease.
PCOS can be a challenge to diagnose because the disorder presents with a wide range of signs and symptoms that can easily be missed (Azziz, 2004). Given the heterogeneity of the disorder, this condition is often under-diagnosed. When a young patient presents with hirsutism and irregular periods, the health care provider should always be alerted to the possibility of PCOS. The diagnostic work-up should be directed toward obtaining a thorough history, performing a physical exam, and ordering laboratory tests of endocrine parameters that will allow the health care provider to make appropriate management decisions that will treat current troubling signs and symptoms. Treatment should also be directed at preventing the long-term health problems commonly seen in women with PCOS.
Widely accepted criteria for diagnosis of PCOS in adolescent patients are based on standards that were established at the 1990 Consensus Conference of the National Institute on Child Health and Human Development (Kent & Legro, 2002). These criteria include chronic anovulation and hyperandrogenism in the absence of other endocrine disorders. The presence of polycystic ovaries is not a criterion for diagnosis in adolescents as it is with young adults and middle age women as determined by the Rotterdam Criteria (2004). History taking, performing the physical examination, and laboratory testing require a focused approach to determine if the patient meets the criteria for diagnosis.
The history should first focus on several aspects regarding menstruation such as (a) age at menarche, (b) length of time between periods, (c) quantity of menstrual flow, and (d) presence of dysmenorrhea (Markle, 2001). Girls with PCOS often begin menstruating with menstrual cycles that are fairly regular. After a few years, the menstrual cycles will become quite irregular or not occur at all. The menstrual flow in girls with PCOS tends to be heavier and is associated with significant cramping.
Obtaining information regarding the development of secondary sexual characteristics is also an important component of the history (Goolsby, 2001). Research has indicated that adolescent girls diagnosed with PCOS had a history of precocious pubarche (Ibanez et al., 2004). Precocious pubarche is defined as the appearance of pubic hair prior to age eight.
Growth charts need to be followed longitudinally with these girls. Often adolescents have had no prior history of obesity as a child but gain a significant amount of weight at an accelerated rate following menarche (Richardson, 2003).
Manifestations of hyperandrogenism need to be explored. Areas to focus on include development of acne, hirsutism, balding, and voice changes (Markle, 2001).
Family history as it relates to PCOS and diabetes must also be explored. PCOS tends to cluster in families and to follow the trend of first degree relatives, especially mothers and sisters of girls diagnosed with the condition (Kashar-Miller, Nixon, Boots, Go, & Azziz, 2001). A family history of diabetes also tends to show up in adolescents with PCOS who are also overweight with insulin resistance and glucose intolerance.
Finally, a social history should be completed. Areas to focus on include current diet and exercise patterns. Because metformin may be one of the medications prescribed, adolescents should also be asked about patterns of alcohol consumption and tobacco use. Alcohol must be avoided when on metformin because excessive alcohol intake is associated with an increased incidence of lactic acidosis; it can potentiate the effects of metformin on lactate. This knowledge will assist the nurse in developing a teaching plan for the adolescent.
Physical examination should begin with measurement of height and weight that will allow for the calculation of body mass index (BMI). If the BMI indicates that the patient is obese, the health care provider should obtain a waist-to-hip ratio to determine if the obesity is considered central/android. An obtained value of greater than 0.72 indicates this type of obesity.
The health care provider would then check for signs indicating hyperandrogenism. The presence of hirsutism, acne, loss of hair, deepening voice, and clitoromegaly are all physical signs indicating a hyperandrogenic state. Acne alone does not indicate PCOS, but it is certainly a sign to look out for as part of the constellation of signs with which an adolescent may present.
Finally, signs of insulin resistance should be noted. The presence of acanthosis nigricans and an increased BMI with the presence of central/android obesity indicate insulin resistance. Acanthosis nigricans is a fairly reliable clinical marker or indicator of moderate to severe insulin resistance. This skin manifestation appears as a brown darkening of the skin at the nape of the neck that will spread laterally to the upper thorax and shoulders. This darkened, velvety-appearing skin can also be noted in the axillary and intertrigenous areas.
Laboratory data are of key importance to confirming the diagnosis, although the history and the outward signs seen on physical exam can lead to the diagnosis of PCOS. Presently, there is no consensus on what biochemical laboratory tests should be used to confirm a diagnosis of PCOS. Most authorities agree that testing should (a) rule out other etiologies of amenorrhea such as hypothyroidism, hyperprolactinemia, and pregnancy; (b) exclude other causes of hyperandrogenism such as congenital adrenal hyperplasia (CAH), as well as adrenal and ovarian tumors; and (c) detect the presence of insulin resistance, glucose intolerance, and lipid abnormalities (Chang, 2004). At present, recommendations for testing should include free testosterone, dehydroepiandrosterone-sulfate (DHEA-S), androstenedione, prolactin, thyroid stimulating hormone (TSH), 17-hydroxyprogesterone (17-OHP), and a pregnancy test. These tests can effectively help the health care provider to (a) rule out other serious problems that may be causing symptoms, (b) make a definitive diagnosis of PCOS, and (c) choose appropriate management strategies and subsequently assess their effectiveness (Chang, 2004; Meisler, 2002; Richardson, 2003; Sheehan, 2004). See Table 1 for a summary of laboratory testing.
Because of the connection between insulin resistance and PCOS, the recommendation is that all adolescents who are suspected of having the disorder be screened by drawing fasting blood glucose levels. If fasting blood glucose levels are elevated, the next step is to order a 2 hour 75 g glucose tolerance test (GTT) to confirm glucose intolerance or type 2 diabetes (Meisler, 2002). In fact, some authorities recommend GTT as a screening test in all obese adolescent females. Testing insulin levels to determine insulin resistance is not recommended because of the time consuming nature of the testing and the expense (Richardson, 2003).
If the adolescent is determined through laboratory testing to have glucose intolerance or type 2 diabetes, ordering a lipid panel would be judicious. Disturbances in glucose metabolism can cause lipid abnormalities, particularly increased total cholesterol, decreased HDL, and increased LDL. Determining baseline lipid levels is also important because many of the treatments, specifically combined oral contraceptive pills (COCPs), can also alter these values and may impact on the provider's choice of treatment modalities.
Patients, depending upon where they fall on the lifespan trajectory, have different issues or concerns related to management of PCOS. Because of the long-term consequences associated with insulin resistance and impaired glucose tolerance related to the development of type 2 diabetes and subsequently cardiovascular disease, it is important to diagnose and treat young girls with this disorder. In addition, because of the body image issues that plague adolescents and their need to be similar to members of their peer group, the hirsutism, acne, weight, and irregularity issues can be disturbing to young women with this disease. Therefore, management strategies for the adolescent patient should focus on resolving irregularity issues regarding the menstrual cycle, controlling hirsutism, managing acne, managing weight, controlling insulin resistance and hyperinsulinemia, and reducing cardiovascular risk factors. The cornerstones of this approach involve both the use of medications and lifestyle modification. In addition, nurses who care for adolescents with the disease must also address hair removal and the psychosocial issues that can be associated with PCOS.
Currently, there are no FDA approved medications indicated for the treatment of PCOS. Any drug that is used in clinical practice is used off-label (Meisler, 2002). Medications commonly prescribed to treat the many troubling signs and symptoms associated with PCOS include oral contraceptive pills, progestin, metformin, AldactoneAE, and Vaniqa99. See Table 2 for a summary of commonly used medications in the treatment of PCOS along with information regarding their use.
For many years, oral contraceptive pills have been the mainstay of therapy for women with PCOS not desiring pregnancy. The best choices are the combined oral contraceptives (COCs). This hormonal therapy can be used to regulate grossly irregular cycles, as well as decrease testosterone, thus decreasing the occurrence of acne and hirsutism. A newer COC, YasminAE, is being marketed as a good choice for PCOS patients because its progestin component (drospirenone) is an analog of spironolactone, a known antiandrogenic agent. An observational study on Yasmin in the management of PCOS symptoms revealed that this COC was effective in improving acne, showed little effect on improving hirsutism, and had a negative influence on fasting insulin concentrations and triglycerides (Palep-Singh, Mook, Barth, & Balen, 2004).
If girls cannot tolerate a COC, another pharmacological method available for regulating cycles is the use of a progestin such as medroxyprogesterone acetate (Provera). The patient is instructed to take 10 mg by mouth daily for 10 days. Once stopped, the patient should have bleeding mimicking a menstrual period. This type of cycle control can be used every 2-3 months to prevent the build up of the endometrial lining of the uterus that may lead to endometrial hyperplasia and subsequent endometrial carcinoma.
Another pharmacological treatment modality that has been used with success, although still being tested through additional clinical trials, is metformin. Metformin HCl (Glucophage) is a biguanide used in the management of patients with type 2 diabetes to control blood glucose levels by improving glucose uptake by peripheral tissues. Most recently, this drug has been used with very good success in women with PCOS to regulate periods, improve ovulation, enhance the effectiveness of fertility drugs, and decrease BMI (Sheehan, 2004). Only recently has the use of metformin with adolescents been studied. Recent clinical trials show good results with altering hyperinsulinemic insulin resistance (Ibanez et al., 2004). In clinical trials of less than 6 months with adolescents, metformin has also been shown to restore normal menstrual cycles (Kent & Legro, 2002). Metformin is prescribed in doses of 1,500 to 2,000 mg daily in treating patients with PCOS. Common gastrointestinal side effects (nausea, vomiting, diarrhea, and flatulence) can be avoided if the medication is started at lower doses and titrated upwards slowly.
Aldactone is another medication used in patients with PCOS. Aldactone (Spironolactone) is actually a diuretic that is commonly used to treat patients with hypertension and heart failure. Aldactone acts as an antiandrogen and actually decreases the amount of free, circulating testosterone, thus impacting most significantly on hirsutism (Richardson, 2003). Up to 200 mg can be taken daily as tolerated. Common side effects include nausea, menstrual irregularities, and increased breast size. The drug needs to be used for at least 6-9 months before any noticeable results may be seen (Attaran, 2005). Once the drug is stopped, hair growth will resume (Tweedy, 2000).
COCs can be used alone or along with antiandrogens and insulin sensitizing agents to improve control of unwanted body hair. In fact, because of the teratogenic effects of aldactone and because metformin can induce ovulatory cycles, if these medications are used, all sexually active females should be encouraged to take a COC concurrently.
Another medication that has shown moderate results in controlling hirsutism is Eflornithine HCl (Vaniqa). This topical agent (cream) acts to slow terminal hair growth resulting in a mild improvement in the presence of unwanted facial hair. Vaniqa is applied to the face, mainly the upper lip, chin, and side-burn area twice daily to achieve the best results.
Although the use of medications has shown promise in the treatment of various symptoms associated with PCOS, the role of lifestyle modification can not be underestimated. Lifestyle modification revolves around weight loss through dietary modification and exercise.
Research indicates that even a moderate decrease in weight, as little as 5% from baseline, can improve rates of ovulation and conception by lowering androgen levels, as well as improve the insulin resistance associated with the disorder (Hill, 2003). Adolescents who are overweight or obese at the time of diagnosis should be encouraged to engage in a diet and exercise plan that will aid them in attaining and subsequently maintaining a healthy body weight, not just for overall health but for controlling many issues related to having PCOS.
Although there is not one particular type of diet reported as being the best diet for women with PCOS, simple dietary modifications that girls with PCOS may want to consider as a way to improve signs and symptoms associated with the disease could be considered. Dietary measures that have shown promise in this population include those that limit simple carbohydrates in lieu of complex carbohydrates. Small, frequent meals that combine healthy proteins, fats, and complex carbohydrates should be encouraged because small frequent meals consumed throughout the day help to lower elevated insulin levels (Tufts University, 2001). This type of eating plan helps to modulate the release of insulin when compared with larger meals filled with simple sugars that cause insulin surges.
The consumption of foods high in polyunsaturated fatty acids (PUFAs) has also been shown to be beneficial in patients with PCOS. PUFAs have been shown to modulate blood glucose as well as to control levels of sex hormones (Kasim-Karakas, 2004). PUFAs can be found in oily fish, nuts, nut butters, olive oil, and canola oil.
Moderate physical activity, 30-60 minutes per day, should be the goal of all patients with PCOS. Aerobic exercise through walking, jogging, swimming, or biking should be encouraged.
The presence of unwanted body hair must also be addressed. Women with PCOS find this one of the most troubling aspects of the disease and the one that can impact their feelings as a woman the most severely (Kitzinger & Willmott, 2002).
Electrolysis and laser ablation therapy are the only two methods that claim to be permanent hair removal techniques. Electrolysis and laser therapy can be expensive and painful procedures. It is also important to refer patients to reputable technicians who perform these procedures to reduce the incidence of complications that are often associated with these methods of hair removal (scarring with electrolysis and hypopigmentation with laser ablation therapy). Plucking and waxing are inexpensive methods that can be performed in the privacy of one's home or through salon services. These are much less expensive, as compared with electrolysis and laser therapies, but are by no means a permanent solution to hirsutism. Shaving is yet another option, but many women find this very undesirable.
Offering psychosocial support can be one of the most important aspects of managing this disease. This begins by building positive, supportive relationships with adolescents diagnosed with PCOS. These relationships will allow the adolescent to express her feelings and concerns regarding having a chronic disease whose signs and symptoms can greatly impact one's body image and self-esteem.
Education is another important component of psychosocial support. Through education, the adolescent can become knowledgeable about the disease and available treatment options. The adolescent will then feel empowered to make informed health care decisions on her own behalf. Education can occur through verbal exchanges, the distribution of written materials, and/or access to Internet-based information contained on Web sites. See Table 3 for a list of Internet resources.
The ability to interact and build relationships with other adolescents who have PCOS can also be a source of psychosocial support. The use of face-to-face or web-based support groups could certainly accomplish this management goal.
PCOS is a chronic disease becoming recognized as impacting adolescents at a much greater degree than previously thought. Nurses are in a critical position to provide comprehensive care to adolescents afflicted with the syndrome. Depending upon where these young females are encountered (in-patient hospital unit, school nurse's office, or outpatient clinic) and the role of the nurse (RN or advanced practice nurse), approaches to care may be different. Regardless of the setting or role, essential elements of nursing practice should always include education and emotional support.
Polycystic Ovary Syndrome (PCOS) in the Adolescent Patient: Recommendations for Practice
Pediatr Nurs. 2005;31(5):416-421.
This column shares new ideas, new policies, new understandings behind diseases and interventions, new resources, new issues, and new roles for the pediatric nurse. For more information, contact Janice Selekman, DNSc, RN; Section Editor; Pediatric Nursing; East Holly Avenue Box 56; Pitman, NJ 08071-0056; (856) 256-2300 or FAX (856) 256-2345.